Chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis
Chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis
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DOI：
10.1016/j.jocn.2012.12.033
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发表时间：
2013-12
影响因子：
2
通讯作者：
K. Murata;H. Ishiguchi;R. Ando;H. Miwa;T. Kondo
中科院分区：
文献类型：
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作者：
K. Murata;H. Ishiguchi;R. Ando;H. Miwa;T. Kondo
我们报告一位慢性发炎性脱髓鞘性多发性神经病合并原发性胆汁性肝硬化的病例。除了最小的生化异常，PBC的临床症状没有观察到，我们诊断为无症状PBC的肝活检的结果，我们的病人。虽然患者注意到很少的肌无力，但电生理学研究显示传导速度缓慢和远端延长，正中神经、尺神经和胫神经存在明确的传导阻滞。受干扰的感觉模式是不对称的，感觉神经动作电位未被诱发。根据这些观察，我们诊断该患者患有慢性炎性脱髓鞘性多发性神经病。与PBC相关的神经病变非常罕见。我们必须区分脱髓鞘神经病变与PBC患者的不对称感觉优势神经病变与高免疫球蛋白M滴度，并调查抗线粒体抗体的存在，以排除并发症的无症状PBC。
We report a patient with chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis (PBC). Except for minimal biochemical abnormalities, clinical symptoms of PBC were not observed, and we diagnosed our patient with asymptomatic PBC from the results of a liver biopsy. Although the patient noticed little muscle weakness, an electrophysiological study demonstrated slow conduction velocities and prolonged distal latencies, with definite conduction blocks in the median, ulnar, and tibial nerves. The disturbed sensory pattern was asymmetrical, and sensory nerve action potentials were not evoked. From these observations, we diagnosed this patient with chronic inflammatory demyelinating polyneuropathy. Neuropathy associated with PBC is very rare. We must differentiate demyelinating neuropathy with PBC in patients with asymmetrical sensory dominant neuropathy with high immunoglobulin M titers, and investigate for the presence of anti-mitochondrial antibodies to rule out a complication of asymptomatic PBC.